Background: Non-invasive diagnosis of disease stage in Fabry cardiomyopathy with
multimodality imaging is pivotal when deciding on the appropriate time to initiate enzyme …

Background: Cardiac response to enzyme replacement therapy (ERT) in Fabry disease is
typically assessed by measuring left ventricular mass index using echocardiography or …

Fabry disease (FD) is a rare X-linked lysosomal storage disorder manifesting as progressive
multi-organ accumulation of sphingolipids due to deficiency in the enzyme α-Galactosidase …

Purpose To perform a systematic review and meta-analysis to assess the effect of enzyme
replacement therapy on cardiac MRI parameters in patients with Fabry disease. Materials …

Background—Enzyme replacement therapy with recombinant α-galactosidase A reduces left
ventricular hypertrophy and improves regional myocardial function in patients with Fabry …

Objective Best treatment outcomes in Fabry disease (FD) associated cardiomyopathy can be
obtained when treatment is started as early as possible. The rationale of this study was to …

Background In spite of several research studies help to describe the heart in Fabry disease
(FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure …

Background Cardiovascular sequelae represent a leading cause of mortality in Fabry
disease. Early detection of cardiac involvement is therefore a subject of considerable …

Aims Cardiac involvement, including progressive cardiomyopathy, is common in Fabry
disease and is a leading cause of premature mortality. We sought to determine if tissue …

Abstract Introduction About 50% of Fabry disease (FD) patients develop cardiac
manifestation, also known as Fabry cardiomyopathy. Beside echocardiography, cardiac …